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CASE REPORT
Year : 2014  |  Volume : 4  |  Issue : 3  |  Page : 190-192

Eventration of right hemidiaphragm, right ectopic kidney, intra-abdominal seminoma in cryptorchid testis: A rare combination or a possible sequence?


Department of Surgery, Government Medical College and Rajindra Hospital, Patiala, Punjab, India

Date of Web Publication8-Dec-2014

Correspondence Address:
Dr. Abhinav Mahajan
Room No. 56, Doctors Hostel, Rajindra Hospital, Patiala - 147 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.146444

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  Abstract 

A thirty-year-old fertile man with a history of right-sided cryptorchidism presented to the surgical outpatient department with a right iliac fossa mass of two months duration. On computed tomography scan of the abdomen, diagnosis of malignant testicular mass with right ectopic kidney and eventration of right hemidiaphragm was made. Exploratory laparotomy with surgical resection of mass was done, which was histopathologically diagnosed as seminoma of the right testis. The unique developmental anomalies and associated seminoma made us report this case. These developmental anomalies may be a possible sequence of events or merely a coincidental combination.

Keywords: Cryptorchidism, eventration, ectopic kidney, seminoma, undescended testis


How to cite this article:
Mahajan A, Varma A, Singh M, Singla SP, Singla A. Eventration of right hemidiaphragm, right ectopic kidney, intra-abdominal seminoma in cryptorchid testis: A rare combination or a possible sequence?. Arch Int Surg 2014;4:190-2

How to cite this URL:
Mahajan A, Varma A, Singh M, Singla SP, Singla A. Eventration of right hemidiaphragm, right ectopic kidney, intra-abdominal seminoma in cryptorchid testis: A rare combination or a possible sequence?. Arch Int Surg [serial online] 2014 [cited 2018 Dec 9];4:190-2. Available from: http://www.archintsurg.org/text.asp?2014/4/3/190/146444


  Introduction Top


Cryptorchidism is one of the most common congenital anomalies, occurring in 1-4% of full-term and 1-45% of preterm male neonates. [1] The majority of cases are isolated, with the ratio of nonsyndromic to syndromic cryptorchidism reported as greater than 6:1 in a large cohort. [1] Genetic studies of cryptorchidism suggest that the disease is heritable but that susceptibility is likely polygenic and multifactorial.

The increased risk of testicular germ cell tumor in males with a history of cryptorchidism has been known for many years. Certain subgroups with undescended testis are at increased risk for germ cell tumors, including those with chromosomal defects and other genital anomalies. Orchiectomy should be considered the preferred treatment of cryptorchid testes from puberty to the age of 50. [2]

Clinically, eventration of diaphragm refers to an abnormal elevation of one leaf of an intact diaphragm because of aplasia or atrophy of varying degrees of muscle fibers. [3] Eventration of the diaphragm is a condition in which all or part of the diaphragm is largely composed of fibrous tissue with only a few or no interspersed muscle fibers. It is usually congenital but may be acquired. [4] Partial eventration of the diaphragm occurs virtually on the right side. [5]

The cranial positioning of kidney is rare. It is seen associated with omphalocele, intrathoracic ectopia, and rarely in congenital or traumatic diaphragmatic hernia. When associated with omphalocele, both kidneys are affected and positioned at the level of T10 under the diaphragm. Patients with this anomaly usually have no symptoms referable to the malposition, and urinary drainage is not impaired. Intrathoracic ectopia denotes either a partial or a complete protrusion of the kidney above the level of the diaphragm into the posterior mediastinum. In congenital or traumatic diaphragmatic hernia, kidneys as well as other abdominal organs can advance into the chest cavity. [6] The objective of this case report is to throw light on this possible combination of anomalies in a single individual as a possible sequence and encourage further research on this subject.


  Case Report Top


A thirty-year-old fertile man with a history of right-sided cryptorchidism presented to the surgical outpatient department with a right iliac fossa mass for the past two months. No other relevant history was found. On examination, there was a spherical mass of 10 cm × 8 cm in size, moving well with respiration and not fixed to overlying skin. The right-side scrotum was found to be empty. Chest X-ray showed elevation of right hemidiaphragm [Figure 1]. Ultrasound examination showed a large soft tissue density mass seen in right iliac fossa. Right kidney was not visualized in right renal fossa. On contrast enhanced computed tomogram of the abdomen, a lobulated heterogeneously enhancing soft tissue mass measuring 12 cm × 6 cm was seen in right lower pelvis [Figure 2]. Hypodense areas suggestive of necrotic changes and a few calcified foci were seen in the mass. The mass was displacing ileal loops superiorly and inferiorly reaching up to the bladder. Fat planes with the surrounding structures were well maintained. Right dome of diaphragm was raised reaching T6 level. Beneath the dome lies the right kidney at T9-12 level. Few lymph nodes were seen in aortocaval and para aortic region. Diagnosis of intra-abdominal mass in cryptorchid testis was made. The patient had exploratory laparotomy.
Figure 1: Chest X-ray showing elevation of right hemidiaphragm

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Figure 2: CT scan showing tumor in the right lower pelvis

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Preoperative tumor marker levels were as follows: LDH = 1095 U/L (normal = 230-460 U/L), beta HCG = 14.32 IU/L (normal = 0.5-2.67 IU/L), AFP = 0.899 IU/mL (normal = 0.5-5.5 IU/mL). Exploratory laparotomy was done and tumor was excised [Figure 3]. Histopathological examination revealed seminoma. Postoperative period was uneventful. Tumor marker levels decreased to LDH = 374 U/L, beta HCG = 5.27 U/L, and AFP = 1.11 IU/mL. Postoperative contrast-enhanced computed tomogram of the abdomen showed no residual tumor, and the patient was followed up for three months without evidence of tumor recurrence.
Figure 3: Cut section of the excised tumor

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  Discussion Top


This case presents a rare opportunity to discuss the possibility of a combination of eventration of right hemidiaphragm and ectopic kidney with associated seminoma in cryptorchid testis as a possible sequence. To the best of our knowledge, there are no previous case reports with such a combination. More research is needed to find out if there is any associated genetic locus mutation with such combination or it is only an incidental sequence of events.

Undescended testes are frequently present in diseases associated with reduced androgen production or action such as androgen biosynthetic defects, androgen insensitivity, Leydig cell agenesis, and gonadotropin deficiency disorders that are typically associated with generalized failure of masculinization.

Certain anomalies are associated with increased risk of cryptorchidism, which include cases of classic prune-belly syndrome,  Spigelia More Detailsn hernia, cerebral palsy, arthrogryposis, myelomeningocele, omphalocele, gastroschisis, imperforate anus, posterior urethral valve, and umbilical hernia patients.

Males with cryptorchidism are four to six times more likely to be diagnosed with testicular cancer, but the relative risk (RR) falls to 2.0-3.0 if orchidopexy is performed before puberty; contralateral descended testis is also at slightly increased risk.

In adults, the diagnosis of diaphragmatic eventration can usually be made on standard PA and lateral chest X-ray. In the PA projection, the elevated diaphragm forms a round unbroken line arching from the mediastinum to the costal arch. In some cases, however, there is no way of knowing whether elevation is caused by congenital absence of muscle or by phrenic paralysis. Asymptomatic patients are managed conservatively but patients with symptoms require surgery. Paradoxical movements suggest complete paralysis and if symptomatic, they are a strong indication for surgery.

Cortes and associates [7] identified an association between renal, T10-S5 spinal anomalies and cryptorchidism, with the affected testis on the same side as the renal anomaly in 90% of cases. These data support common origins of cryptorchidism and abnormalities of urogenital ridge, abdominal wall, lumbosacral spine, and CNS development.

A sequence occurs when a single developmental defect results in a chain of secondary defects, which may, in turn, lead to tertiary defects. The result is a variably expressed group of defects, all of which can be traced back to the original event. In the present case, it is very difficult to label it as a sequence as the single primary defect is unclear.

There are cases describing combination of ectopic kidney with cryptorchidism or with eventration of hemidiaphragm. [8] Nobody has described these three conditions with development of seminoma in one patient. This unique case report invites further interest on this combination or possible sequence of events.

 
  References Top

1.
Boyd HA, Myrup C, Wohlfahrt J, Westergaard T, Nørgaard-Pedersen B, Melbye M. Maternal serum alpha-fetoprotein level during pregnancy and isolated cryptorchidism in male offspring. Am J Epidemiol 164:478-86.  Back to cited text no. 1
    
2.
Wood HM, Elder JS. Cryptorchidism and testicular cancer: Separating fact from fiction. J Urol 2009;181:452-61.  Back to cited text no. 2
    
3.
Thomas TV. Congenital eventration of diaphragm. Ann Thorac Surg 1970;10:180-92.  Back to cited text no. 3
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4.
Laxdale OE, McDougall H, Mellin GW. Congenital eventration of the diaphragm. N Engl Med 1954;250:401-8.  Back to cited text no. 4
    
5.
Seaton A. Abnormalities and Diseases of the Diaphragm. In: Seaton A, Seaton D, Leitch AG, editors. Crofton and Douglas's Respiratory Diseases. 5 th ed. Oxford: Blackwell Science; 2000. p. 1234-49.  Back to cited text no. 5
    
6.
Campbell walsh urology. 10 th ed. In: Kavoussi LR, Novick AC, Partin AW, Peters CA, editors. Philadelphia: Saunders, an imprint of Elsevier; 2012. p. 3138.  Back to cited text no. 6
    
7.
Cortes D, Thorup JM, Beck BL, Visfeldt J. Cryptorchidism as a caudal developmental field defect. A new description of cryptorchidism associated with malformations and dysplasias of the kidneys, the ureters and the spine from T10 to S5. Acta Pathologica, Microbiologica, et Immunologica Scandinavica 1998;106:953-8.  Back to cited text no. 7
    
8.
Bulgrin JG, Holmes FH. Eventration of the diaphragm with high renal ectopia; A case report. Radiology 1955;64:249-51.  Back to cited text no. 8
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